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Washington University Experience | MYELIN (IMMUNE-MEDIATED) | Inflammatory Demyelination | 15A0 Case 16 History
Case 15 History ---- On 6/26 the patient developed a sore throat, swollen tonsils, and difficulty swallowing. Strep, mono, COVID and flu testing were negative and the patient received IM solumedrol. Symptoms continued to worsen and she was treated with antibiotics and discharged. She represented on 7/11 reporting a 3 day history of headache, slurred speech, unsteady walking, horizontal binocular diplopia, and right face and bilateral hand numbness. OSH neurology admitted her with concern for MS. A brain MRI on 7/12 showed irregular patchy areas of enhancement and edema mostly in the pons and medulla, but also extending to the left thalamus, left temporal lobe, left periventricular occipital area, and frontoparietal subcortical white matter. There was concern for CLIPPERS following CSF lymphocytosis. LP (7/13) showed elevated nucleated cells (55) and protein (52). Meningitis/encephalitis panel, CMV, VZV, HSV, WNV, JCV were all negative. Bacterial culture was negative. Cytology showed mature lymphocytic pleocytosis. MOG, NMO, ANA, and IgG index were also negative. She was treated with IV solumedrol 1g daily for 5 days (7/12-7/16), with improvement. Repeat MRI on 7/17 showed persistently increased T2 and FLAIR signal of the pons, subcortical and periventricular white matter were also decreased in size and without postcontrast enhancement, DDx c/w ADEM or CLIPPERS, and less likely lymphoma or glioma. The patient was started on a steroid taper on 7/17. She was also started on gabapentin for paresthesias. At the time of discharge (7/20), her mother recalls improvement in speech, ambulation, and fine motor skills. Diplopia and numbness remained persistent and unchanged. On 7/21 she developed worsening slurred speech, drooling and gait instability. Since then, her mother has also noticed that she has seemed more confused and has episodes of "giddiness" and inappropriate laughter. She has also displayed some unusual behaviors such as becoming very fixated on and picking at her IV. Notes from 7/23 document increasing right-sided weakness and ataxia on that day. Prednisone was increased and Gabapentin was also held, but no improvement was noted in her symptoms. An MRI on 7/23 showed improvement and decreasing size of multifocal T2/FLAIR hyperintensities. Residual enhancement was slightly worse compared to her 7/17 MRI, but overall improved from 7/12 MRI. She presented to BJH ED on 7/24 for evaluation. She reports no fever, chills, abdominal pain, nausea, vomiting, diarrhea, dysuria, urgency, cough, rhinorrhea, or dyspnea in the days leading up to this admission. Family History was positive for Multiple Sclerosis in her father who passed away in his 20s and a grandmother with SLE. A broad differential, including inflammatory disorders such as ADEM and CLIPPERS, a neoplastic process such as lymphoma (less likely glioma) vs. sarcoidosis vs. atypical demyelinating syndrome vs. vasculitis/vasculopathy (immune or mycoplasma associated) vs. Histiocytic disorder (e.g. Erdheim Chester).
